Arrhythmogenic cardiomyopathy and differential diagnosis with physiological right ventricular remodelling in athletes using cardiovascular magnetic resonance.


Journal

The international journal of cardiovascular imaging
ISSN: 1875-8312
Titre abrégé: Int J Cardiovasc Imaging
Pays: United States
ID NLM: 100969716

Informations de publication

Date de publication:
Dec 2022
Historique:
received: 18 03 2022
accepted: 22 06 2022
entrez: 29 11 2022
pubmed: 30 11 2022
medline: 2 12 2022
Statut: ppublish

Résumé

To describe the overlap between structural abnormalities typical of arrhythmogenic right ventricular cardiomyopathy (ARVC) and physiological right ventricular adaptation to exercise and differentiate between pathologic and physiologic findings using CMR. We compared CMR studies of 43 patients (mean age 49 ± 17 years, 49% males, 32 genotyped) with a definitive diagnosis of ARVC with 97 (mean age 45 ± 16 years, 61% males) healthy athletes. CMR was abnormal in 37 (86%) patients with ARVC, but only 23 (53%) fulfilled a major or minor CMR criterion according to the TFC. 7/20 patients who did not fulfil any CMR TFC showed pathological finding (RV RWMA and fibrosis in the LV or LV RWMA). RV was affected in isolation in 17 (39%) patients and 18 (42%) patients showed biventricular involvement. Common RV abnormalities included RWMA (n = 34; 79%), RV dilatation (n = 18; 42%), RV systolic dysfunction (≤ 45%) (n = 17; 40%) and RV LGE (n = 13; 30%). The predominant LV abnormality was LGE (n = 20; 47%). 22/32 (69%) patients exhibited a pathogenic variant: PKP2 (n = 17, 53%), DSP (n = 4, 13%) and DSC2 (n = 1, 3%). Sixteen (16%) athletes exceeded TFC cut-off values for RV volumes. None of the athletes exceeded a RV/LV end-diastolic volume ratio > 1.2, nor fulfilled TFC for impaired RV ejection fraction. The majority (86%) of ARVC patients demonstrate CMR abnormalities suggestive of cardiomyopathy but only 53% fulfil at least one of the CMR TFC. LV involvement is found in 50% cases. In athletes, an RV/LV end-diastolic volume ratio > 1.2 and impaired RV function (RVEF ≤ 45%) are strong predictors of pathology.

Identifiants

pubmed: 36445664
doi: 10.1007/s10554-022-02684-y
pii: 10.1007/s10554-022-02684-y
doi:

Types de publication

Journal Article

Langues

eng

Sous-ensembles de citation

IM

Pagination

2723-2732

Informations de copyright

© 2022. The Author(s), under exclusive licence to Springer Nature B.V.

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Auteurs

Eleonora Moccia (E)

Cardiology Clinical Academic Group, St George's University of London and St George's University Hospital NHS Foundation Trust, London, UK. e.moccia@studenti.uniss.it.
Clinical and Interventional Cardiology, Sassari University Hospital, Via Enrico de Nicola 1, Sassari, Italy. e.moccia@studenti.uniss.it.

Efstathios Papatheodorou (E)

Cardiology Clinical Academic Group, St George's University of London and St George's University Hospital NHS Foundation Trust, London, UK.

Chris J Miles (CJ)

Cardiology Clinical Academic Group, St George's University of London and St George's University Hospital NHS Foundation Trust, London, UK.

Ahmed Merghani (A)

Cardiology Clinical Academic Group, St George's University of London and St George's University Hospital NHS Foundation Trust, London, UK.

Aneil Malhotra (A)

Cardiology Clinical Academic Group, St George's University of London and St George's University Hospital NHS Foundation Trust, London, UK.

Harshil Dhutia (H)

Cardiology Clinical Academic Group, St George's University of London and St George's University Hospital NHS Foundation Trust, London, UK.

Rachel Bastiaenen (R)

Cardiology Clinical Academic Group, St George's University of London and St George's University Hospital NHS Foundation Trust, London, UK.
Guy's and St, Thomas's Hospital, London, UK.

Nabeel Sheikh (N)

Cardiology Clinical Academic Group, St George's University of London and St George's University Hospital NHS Foundation Trust, London, UK.
Guy's and St, Thomas's Hospital, London, UK.

Abbas Zaidi (A)

Cardiology Clinical Academic Group, St George's University of London and St George's University Hospital NHS Foundation Trust, London, UK.
University Hospital of Wales, Cardiology, Cardiff, UK.

Giuseppe Damiano Sanna (GD)

Clinical and Interventional Cardiology, Sassari University Hospital, Via Enrico de Nicola 1, Sassari, Italy.

Tessa Homfray (T)

Cardiology Clinical Academic Group, St George's University of London and St George's University Hospital NHS Foundation Trust, London, UK.

Nicholas Bunce (N)

Cardiology Clinical Academic Group, St George's University of London and St George's University Hospital NHS Foundation Trust, London, UK.

Lisa J Anderson (LJ)

Cardiology Clinical Academic Group, St George's University of London and St George's University Hospital NHS Foundation Trust, London, UK.

Maite Tome (M)

Cardiology Clinical Academic Group, St George's University of London and St George's University Hospital NHS Foundation Trust, London, UK.

Elijah Behr (E)

Cardiology Clinical Academic Group, St George's University of London and St George's University Hospital NHS Foundation Trust, London, UK.

James Moon (J)

Barts Heart Centre, St Bartholomew's Hospital, London, UK.
Institute of Cardiovascular Science, University College London, London, UK.

Sanjay Sharma (S)

Cardiology Clinical Academic Group, St George's University of London and St George's University Hospital NHS Foundation Trust, London, UK.

Gherardo Finocchiaro (G)

Cardiology Clinical Academic Group, St George's University of London and St George's University Hospital NHS Foundation Trust, London, UK.
Guy's and St, Thomas's Hospital, London, UK.

Michael Papadakis (M)

Cardiology Clinical Academic Group, St George's University of London and St George's University Hospital NHS Foundation Trust, London, UK.

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